The Management of Swallowing Disorders in Children With Spinal Muscular Atrophy Type 1: A Scoping Review.

Purpose

Spinal muscular atrophy Type 1 (SMA1) is a rare autosomal recessive neuromuscular disorder with progressive muscle weakness and atrophy. Dysphagia is common among children with SMA1, and its complications are frequently documented. This scoping review aimed to describe the management of swallowing disorders in this population.

Method

Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, four electronic databases were searched (PubMed, Embase, CINAHL, and Cochrane Library) for studies on children with SMA1. We investigated the management of swallowing disorders in children with SMA1, including methods and protocols used to assess swallowing and feeding, and treatment option. The quality of included studies was assessed using the Joanna Briggs Institute checklists; two independent researchers conducted study selection and data extraction.

Results

Thirty-eight studies with a total of 1,784 SMA1 patients were included. Swallowing was evaluated using instrumental methods (flexible endoscopic evaluation of swallowing and videofluoroscopic swallowing study) and clinical assessments, with one validated protocol identified for pediatric SMA (Oral and Swallowing Abilities Tool). Delayed initiation of swallowing, oral impairment, pharyngeal residues, and aspiration were frequently reported. Percutaneous endoscopic gastrostomy was used in cases of severe dysphagia, malnutrition, and failure to thrive. Few studies reported information on compensatory strategies and rehabilitative treatments.

Conclusion

Further research is needed to standardize swallowing assessment approaches and to evaluate the effectiveness of rehabilitative and compensatory interventions in children with SMA1. SUPPLEMENTAL MATERIAL https://doi.org/10.23641/asha.33746656.